The Adrenal Glands

Penyakit Endokrin – Gangguan hormonal

Hipofise
Tiroid
Paratiroid
Adrenal
Patologi Anatomi
Patologi Klinik
The Adrenal Glands
 SKDI 2012 endokrin
• Diabetes melitus tipe 1 4A • Hiperparatiroid 1
• Diabetes melitus tipe 2 4A • Hipoparatiroid 3A
• Diabetes melitus tipe lain 3A • Hipertiroid 3A
(intoleransi glukosa akibat • Tirotoksikosis 3B
penyakit lain atau obat-obatan) • Hipotiroid 2
• Ketoasidosis diabetikum 3B • Goiter 3A
• Hiperglikemi hiperosmolar 3B • Tiroiditis 2
non ketotik • Cushing's dis 3B
• Hipoglikemia ringan 4A • Krisis adrenal 3B
• Hipoglikemia berat 3B • Addison's dis 1
• Diabetes insipidus 1 • Pubertas prekoks 2
• Akromegali, gigantisme 1 • Hipogonadisme 2
Hemianopsia bilateral • Prolaktinemia 1
• Defisiensi hormon 1 • Adenoma tiroid 2
pertumbuhan • Karsinoma tiroid 2
 Case report
A 10-year-old girl has increased serum testosteron,
decreased aldosterone, and increased ACTH.
Her testosterone decrease after given low-dose
dexamethasone.
Her adrenal would appear as shown (bottom)
compared with a normal organ (top).
• What gene abnormality is lakely present ?
• Explain the laboratory findings.
• How might this be treated ?

Sumber : Study companion for Robbins and Cotran Pathology, 2010

 Adrenal
Sumber : Study companion for Robbins and Cotran Pathology, 2010
A 5-year-old boy has developed feature suggestive
over the past 6 months.
On physical examination, the boy has secondary sex
characteristic including pubic hair and enlargement
of the penis. Which of the following morphologic
features is the most likely to be seen in his adrenal
glands ?
A. Bilateral adrenal cortical hyperplasia
B. Bilateral adrenal cortical atrophy
C. Nodule in the adrenal medulla
D. Normal size and architecture
E. Nodule in adrenal cortex
 The Adrenal Glands#1
• Are paired endocrine organs consist of cortex & medulla
(differ in development, structure & function).
Cortex consists of 3 layers of distinct cell types;
1) Beneath the capsule of adrenal is narrow layer
of zona glomerulosa.
2) An equally narrow zona reticularis abuts medulla.
3) Intervening is the broad zona fasciculata
(±75% of total cortex).
3 zona di daerah korteks Kelenjar adrenal
• Zona glomerulosa – hormon mineralokortikoid
• Zona fasikulata Glukokortikoid
• Zona retikularis
Medula – katekolamin

Mineralokortikoid (Aldosteron) bersama dengan sistim renin-

angiotensin & ADH  menjaga volume darah
Glukokortikoid (kortisol & kortikosteron)
• Merangsang glukoneogenesis  gula darah
• Menghambat sintesis protein
• Anti inflamasi, limfotoksik, menekan GH
Hormon sex korteks adrenal (testosteron)
• Feminisasi wanita dihambat
• Pubertas prematur
 The Adrenal Glands#2
Adrenal cortex synthesizes
3 different types of steroids:
1) Glucocorticoids (principally
cortisol), which are synthesize
primarily in zona fasciculata with
a small contribution from zona
reticularis.
2) Mineralocorticoids (most
important aldosterone),
which is generated in zona
glomerulosa.
3) Sex steroids (estrogens &
androgens), which are produced
largely in zona reticularis.
 The Adrenal Glands#3
• The adrenal medulla is composed of chromaffin
cells, which synthesize & secrete catecholamines
(mainly epinephrine) .
 The Adrenal Glands#3
Diseases of adrenal cortex can be divided into;
•Those associated with cortical hyperfunction
•Those characterized by cortical hypofunction
 Kelainan fungsi adrenal

Sindroma Cushing

Hiperfungsi Sindroma Conn

Hipersekresi ho sex korteks

Krisis adrenal (akut)

Hipofungsi Penyakit Addison (kronik)

Insufisiensi sekunder
Akibat keadaan kortisol >> Sindroma Cushing
• Iatrogenik (>>)
- Glukokortikoid / ACTH dosis tinggi lama
• Adenoma / karsinoma korteks
- Kadar kortisol serum tp ACTH normal
• Sekresi ACTH >> oleh HF
- Dewasa >>
- Hiperplasia sel adrenal
- Ditekan dng deksametason
• Sekresi ACTH oleh tumor non HF
- Oat cell Ca pd bronkus, karsinoid, timus, pankreas
- Tak dapat ditekan dng deksametason
Bedakan dengan peny. Cushing (Adenoma sel basofil HF)
Figure 20-35 Schematic representation of the various forms of Cushing syndrome,
illustrating the three endogenous forms, as well as the more common
exogenous (iatrogenic) form. ACTH, adrenocorticotropic hormone.
The signs and symptoms of Cushing syndrome
• Hypercortisolism causes selective atrophy of fast-twitch
(type II) myofibers, with resultant
decreased muscle mass & proximal limb weakness.
• Glucocorticoids induce gluconeogenesis & inhibit
the uptake of glucose by cells, with resultant
hyperglycemia, glucosuria & polydipsia, mimicking
diabetes mellitus.
• The catabolic effects on proteins cause loss of collagen &
resorption of bone. Thus, the skin is thin, fragile & easily
bruised; cutaneous striae are particularly common in
abdominal area.
• Bone resorption results in the development of
osteoporosis, with consequent increased susceptibility to
fractures.
The signs and symptoms of Cushing syndrome
• Because glucocorticoids suppress the
immune response, patients with
Cushing syndrome are also at increased
risk for a variety of infections.
• Additional manifestations include
hirsutism & menstrual abnormalities, as
well as number of mental disturbances,
including mood swings, depression &
frank psychosis.
• Extra-adrenal Cushing syndrome caused
by pituitary or ectopic ACTH secretion
is usually associated with increased skin
pigmentation, because of
melanocyte-stimulating activity in ACTH
precursor molecule.
 Hiperadrenalisme Penyebab
Glukokortikoid / kortisol • Hiperplasia adrenal
• Neoplasma adrenal
• Terapi kortikosteroid
Kelainan :
• Obesitas sentral (eunuch)
• Moon face
• Kelemahan otot
• Osteoporosis
• Atrofi kulit, stria merah
• Toleransi glukosa
• Hipertensi
• Hirsutisme, kelainan haid
• Hipokalemi
 Hiperadrenalisme
mineralokortikoid - aldosteron
Penyebab :
• Adenoma zona glomerulosa
Mekanisme :
• Sistim renin-angiotensin
Kelainan :
• Hipertensi
• Hipokalemi
• Sodium sedikit meningkat
• Aldosteron plasma naik
Hiperadrenalisme hormon sex
• Menyebabkan sindroma
adrenogenital
• Def. enz 21-hidroksilase
Androgen / testosteron >>
• Maskulinisasi wanita
• Pubertas prekoks & feminisasi
Sindr adrenogenital ada 3 btk
• Salt wasting
• Virilisme sederhana
• Virilisme klasik
 Hipofungsi adrenal
Krisis adrenal
Insufisiensi akut korteks adrenal
Akibat kegagalan meningkatkan steroid secara cepat
setelah :
• Operasi adrenalektomi
• Septikemia / bakteriemia  destruksi masif korteks
(Sindr Waterhouse-Friderichsen)
• Syok endotoksin
• Destruksi masif korteks adrenal pd neonatus akibat
partus lama
 Hipofungsi cortex adrenal
Penyakit Addison
Insufisiensi kronik primer
• O.k otoimun, TBC & metastasis Ca
• Lemah, anoreksia
• BB turun, hipotensi
• Eksaserbasi akut mjd krisis adrenal karena
infeksi, diet, vomitus, diare
Insufisiensi sekunder korteks adrenal
• Akibat kegagalan HF mensekresi ACTH
• Gambaran klinik = hipopituitarisme
 ADRENAL INSUFFICIENCY
Adrenocortical insufficiency (hypofunction) caused;
• Primary adrenal disease (primary hypoadrenalism)
• Decreased stimulation of adrenals resulting from a deficiency
of ACTH (secondary hypoadrenalism).
The patterns of adrenocortical insufficiency can be
considered under the following headings:
(1)Primary acute adrenocortical insufficiency (Adrenal crisis)
(2)Primary chronic adrenocortical insufficiency (Addison
disease)
(3)Secondary adrenocortical insufficiency.
 Krisis adrenal
Penyebab
• Bacterial septicemia
• Toksin bakteri menyebabkan kerusakan difus
vaskuler  DIC & perdarahan  merusak
korteks adrenal
• Trauma lahir
• Pengobatan antikoagulan
• Semua penyebab DIC
Akibat : Glukokortikoid menurun mendadak
Figure 20-39 Acute adrenal insufficiency caused by severe bilateral adrenal hemorrhage in an infant with overwhelming sepsis
(Waterhouse-Friderichsen syndrome). At autopsy the adrenals were grossly hemorrhagic and shrunken; microscopically, little residual
cortical architecture is discernible.
 Neoplasma

Korteks Adenoma

Neuroblastoma
Ganglioneuroma
Medula
Phaeochromocytoma
 Case report
A 10-year-old girl has increased serum testosteron,
decreased aldosterone, and increased ACTH.
Her testosterone decrease after given low-dose
dexamethasone.
Her adrenal would appear as shown (bottom)
compared with a normal organ (top).
• What gene abnormality is lakely present ?
• Explain the laboratory findings.
• How might this be treated ?

Sumber : Study companion for Robbins and Cotran Pathology, 2010

 Adrenal
Sumber : Study companion for Robbins and Cotran Pathology, 2010
A 5-year-old boy has developed feature suggestive
over the past 6 months.
On physical examination, the boy has secondary sex
characteristic including pubic hair and enlargement
of the penis. Which of the following morphologic
features is the most likely to be seen in his adrenal
glands ?
A. Bilateral adrenal cortical hyperplasia
B. Bilateral adrenal cortical atrophy
C. Nodule in the adrenal medulla
D. Normal size and architecture
E. Nodule in adrenal cortex
 Answers
1. In this form of congenital adrenal hyperplasia (CAH),
the functional CYP21B gene on chromosome 6 is
typically recombined with (and replaced by) an adjacent
non functional psudogene CYP21A.
2. The lack of adrenal 21-hydroxylase activity and dificient
mineralocorticoid production leads to salt wasting and
hypotension with hyperkalemia ; deficient cortisol
production leads to elevated ACTH that drives adrenal
hyperplasia with overproduction of sex steroids.
3. Exogenous glucocorticoids replace the cortisol
requirement and supress the ACTH overproduction ;
mineralocrticoids must also be given in the salt wasting
form of CAH.

Sumber : Study companion for Robbins and Cotran Pathology, 2010